Adiposis dolorosa

Adiposis dolorosa
Adiposis dolorosa
Classification and external resources
ICD-10 E88.2
ICD-9 272.8
OMIM 103200
DiseasesDB 29660
eMedicine derm/839
MeSH D000274

Adiposis dolorosa, also known as Dercum's disease[1] is a rare condition characterized by multiple, painful lipomas. These lipomas mainly occur on the trunk, the upper arms and upper legs. The diagnosis of Dercum's disease implies a long, chronic pain syndrome of debilitating nature. The exact cause of Dercum’s disease is unknown.


Contents

History

Dercum's disease was originally described in the medical literature in 1892 by the American doctor Frances Xavier Dercum.[2]

Symptoms

Multiple painful lipomas is the cardinal symptom of this disease. The pain can be very intense and can be described as aching, stabbing, smarting or burning. The pain is chronic and progressive, but varies much in cycles.

Other common symptoms are:

  • Various areas of the body may swell for no apparent reason. The fingers becomes clumsy, a person may drop things and sometimes the fingers go numb.
  • Disturbed sleep, many have difficulties going to sleep because of the pain, but other forms of sleeping disorders also occur.
  • General fatigue, worsening with even mild activity.
  • Tendency to become black and blue; this seems to arise spontaneously or after alleviated blow.
  • Stiffness after resting especially in the mornings.
  • Skeletal pain in wrist, elbows, hips, tail bone and the long bones of the arms and legs.
  • Headache, usually a combination between tension headache and classic migraine.
  • Memory lapses and concentration difficulties making it difficult to learn new things and to accomplish intellectually demanding jobs.
  • Feeling hot is often felt by the patients, some have 37.5 to 39 Celsius degree fever several weeks in a row, with increased pain and incapacity to work as a consequence.
  • Tenderness under the feet, akin to walking on glass.
  • Tenderness in the skin, difficulties in wearing tight fitting clothes or taking a shower.
  • Infection sensitivity. Frequently increased pain during infections or active allergy attacks.

Causes

The cause of the disease is unknown and poorly researched. The symptoms and the lack of treatments can seriously affect the quality of life.

Diagnosis

A diagnosis of Dercum's disease is based on patient history and the physical findings. There are no specific laboratory test for this disease. Since the disorder does not show on the outside it can be hard for others to see the level of difficulties and discomfort the patient experiences. More than half of the patients are unable to work. The knowledge about the disorder is not well known and many patients are poorly treated. Some lab tests are currently being used to aid in the diagnosis but are not diagnostic themselves. Ultrasound and MRI are also useful in seeing the nodules found in visceral fat. Magnetic resonance imaging can play a role in diagnosis.[3]

Treatment

Treatment of Dercum's disease is usually targeted towards pain relief rather than removal. Currently, there is a lack of scientific data on the use of integrative therapies for the treatment or prevention of Dercum's disease. [4] There are not enough studies done to prove that diet and supplements could help with the disease.[5]

Treatment methods include:

Surgery

  • Surgical excision of fatty tissue deposits around joints (liposuction) has been used in some cases.[6] It may temporarily relieve symptoms although recurrences often develop.

Medication

  • Fentanyl
  • Cannabis
  • Lyrica
  • The use of infliximab and methotrexate has been proposed.[7]
  • Intravenous infusions of the local anaesthetic drug lidocaine may give temporary relief from pain.[8] Additional treatments of lidocaine may be necessary periodically to sustain the effect.
  • Another analgesic drug, peroral mexiletine, may also be effective in eliminating pain for variable periods of time.[9]

References

  1. ^ synd/1453 at Who Named It?
  2. ^ Dercum, F. X. Three cases of a hitherto unclassified affection resembling in its grosser aspects obesity, but associated with special nervous symptoms: adiposis dolorosa. Am. J. Med. Sci. 104: 521-535, 1892.
  3. ^ Amine B, Leguilchard F, Benhamou CL (March 2004). "Dercum's disease (adiposis dolorosa): a new case-report". Joint Bone Spine 71 (2): 147–9. doi:10.1016/S1297-319X(03)00139-8. PMID 15050201. http://linkinghub.elsevier.com/retrieve/pii/S1297319X03001398. 
  4. ^ [1] Natural Standard Research Collaboration
  5. ^ [2] Rowena Santos, MD
  6. ^ De Silva M, Earley MJ (June 1990). "Liposuction in the treatment of juxta-articular adiposis dolorosa". Ann. Rheum. Dis. 49 (6): 403–4. doi:10.1136/ard.49.6.403. PMC 1004110. PMID 2383065. http://ard.bmj.com/cgi/pmidlookup?view=long&pmid=2383065. 
  7. ^ Singal A, Janiga JJ, Bossenbroek NM, Lim HW (May 2007). "Dercum's disease (adiposis dolorosa): a report of improvement with infliximab and methotrexate". J Eur Acad Dermatol Venereol 21 (5): 717. doi:10.1111/j.1468-3083.2006.02021.x. PMID 17448013. http://www.blackwell-synergy.com/openurl?genre=article&sid=nlm:pubmed&issn=0926-9959&date=2007&volume=21&issue=5&spage=717. 
  8. ^ Devillers AC, Oranje AP (May 1999). "Treatment of pain in adiposis dolorosa (Dercum's disease) with intravenous lidocaine: a case report with a 10-year follow-up". Clin. Exp. Dermatol. 24 (3): 240–1. doi:10.1046/j.1365-2230.1999.00466.x. PMID 10354189. http://www.blackwell-synergy.com/openurl?genre=article&sid=nlm:pubmed&issn=0307-6938&date=1999&volume=24&issue=3&spage=240. 
  9. ^ Steiner J, Schiltz K, Heidenreich F, Weissenborn K (February 2002). "[Lipomatosis dolorosa--a frequently overlooked disease picture]" (in German). Nervenarzt 73 (2): 183–7. doi:10.1007/s00115-001-1240-9. PMID 11975097. 

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Look at other dictionaries:

  • adiposis dolorosa — Véase enfermedad de Dercum. Diccionario Mosby Medicina, Enfermería y Ciencias de la Salud, Ediciones Hancourt, S.A. 1999 …   Diccionario médico

  • adiposis dolorosa — adiposis do·lo·ro·sa .dō lə rō sə, zə n a condition of generalized obesity characterized by pain in the abnormal deposits of fat * * * nodular circumscribed lipomatosis in which the adipose deposits are tender or painful because of pressing… …   Medical dictionary

  • adiposis dolorosa — n. (Medicine) Anders disease, adiposis tuberosa simplex, disease characterized by painful localized fatty masses or cutaneous nodules (on the abdomen or extremities) that are sensitive to touch, lipomatosis …   English contemporary dictionary

  • adiposis dolorosa — ˌdōləˈrōsə noun Etymology: New Latin, literally, painful adiposis : a condition of generalized obesity characterized by pain in the abnormal deposits of fat …   Useful english dictionary

  • dolorosa — see ADIPOSIS DOLOROSA …   Medical dictionary

  • adiposis — Excessive local or general accumulation of fat in the body. SYN: lipomatosis, liposis (1), steatosis (1). [adipo + G. osis, condition] a. cerebralis obesity resulting from intracranial disease, most commonly of the hypothalamus, r …   Medical dictionary

  • adiposis tuberosa simplex — a disorder resembling adiposis dolorosa, marked by development in the subcutaneous tissue of fatty masses that may be painful to pressure …   Medical dictionary

  • adiposis tuberosa simplex — n. (Medicine) Anders disease, adiposis dolorosa, disease characterized by painful localized fatty masses or cutaneous nodules (on the abdomen or extremities) that are sensitive to touch, lipomatosis …   English contemporary dictionary

  • adiposis — liposis; n. the presence of abnormally large accumulations of fat in the body. The condition may arise from overeating, hormone irregularities, or a metabolic disorder. In adiposis dolorosa, a condition affecting women more commonly than men,… …   The new mediacal dictionary

  • Lipomatosis dolorosa — Klassifikation nach ICD 10 E88.2 Lipomatose, anderenorts nicht klassifiziert Lipomatosis dolorosa [Dercum Krankheit] …   Deutsch Wikipedia

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